AUTHOR=Wang Qianwen , Wu Kangze , Zhang Xuzhao , Liu Yang , Sun Zhouyi , Wei Shumei , Zhang Bo 

TITLE=Primary hepatopancreatobiliary lymphoma: Pathogenesis, diagnosis, and management

JOURNAL=Frontiers in Oncology

VOLUME=Volume 12 - 2022

YEAR=2022

URL=https://www.frontiersin.org/journals/oncology/articles/10.3389/fonc.2022.951062

DOI=10.3389/fonc.2022.951062

ISSN=2234-943X

ABSTRACT=Primary hepatopancreatobiliary lymphoma (PHPBL) is extremely rare, which is defined as a lympho-proliferative disease confined to hepatobiliary and pancreas without any involvement of lymph nodes, bone marrow or other organs. The clinical and imaging manifestations of PHPBL are variable and non-special, which are akin to those of tumor of hepatobiliary and pancreatic system. The overall prognosis and management of PHPBL differ from those of other tumors in hepatobiliary and pancreas. Proper diagnosis and prompt treatment are essential for improving clinical outcomes. Due to its rarity, the optimal treatment has not been issued. However, CHOP/R-CHOP-based chemotherapy is considered as a standard treatment for them. This review provides an overview of the pathogenesis, diagnosis, pathology, and management of PHPBL and offer clinicians the diagnosis and management schedule for PHPBL.